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Several different types of cancer can affect the small intestines. Doctors classify these cancers based on the type of cells that are affected. About 15 percent to 20 percent of intestinal cancers are lymphomas. Intestinal lymphomas usually occur in adults who are at least 60 years old and are slightly more likely to develop in males than in females.
Lymphoma is cancer that develops from lymphocytes (a type of white blood cell). There are two main types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma (NHL).
Lymphoma affects the organs and tissues of the lymphatic system, which helps the body fight infection and get rid of waste. Someone with lymphoma usually has too many lymphocytes collecting in the lymph nodes in their neck, armpits, or groin. Sometimes, lymphoma may also occur in extranodal sites (areas outside the lymph nodes). When lymphoma begins in one of these other locations, it is considered primary extranodal lymphoma.
About 30 percent to 40 percent of extranodal lymphomas develop in the gastrointestinal (GI) tract (digestive system). Almost all GI lymphomas are a type of non-Hodgkin lymphoma. Most GI lymphomas affect the stomach. The small intestines, also called the small bowels, are the second most common spot for GI lymphomas to develop. Lymphoma can develop in any part of the small intestine: the duodenum, jejunum, or ileum. Gastrointestinal lymphomas may also affect a person’s throat, esophagus (tube that connects the mouth to the stomach), colon (large intestine), and rectum.
Beyond the main types of intestinal lymphoma — Hodgkin lymphoma and non-Hodgkin lymphoma — the disease is divided into subtypes based on the cells they affect. About 9 out of 10 GI lymphomas affect a type of lymphocyte called B cells. Rarely, GI lymphomas may affect someone’s T cells (another type of lymphocyte).
Intestinal B-cell lymphomas include:
There are two kinds of intestinal T-cell lymphomas. The first is called “enteropathy-associated T-cell lymphoma (EATL).” It occurs only in people who have celiac disease. (It was previously referred to as “EATL type 1.”)
The second is called “monomorphic epitheliotropic intestinal T-cell lymphoma.” Researchers say this kind of lymphoma — previously called EATL type 2 — is more likely to develop in people who are Asian or Hispanic.
Intestinal lymphoma often causes signs and symptoms such as:
T-cell intestinal lymphoma may also cause an itchy rash. It also sometimes leads to what are called “B symptoms.” These symptoms include things like fevers, night sweats, and a loss of more than 10 percent of a person’s body weight.
Intestinal lymphoma can cause potentially serious medical problems. In one study of more than 1,000 people with the condition, nearly 1 out of 9 developed a hole (perforation) in their intestines. Intestinal perforations can be serious. Some people with intestinal lymphoma stay in the hospital when they first start treatment so their doctors can notice possible signs of perforation right away. These holes can lead to fever, chills, extreme abdominal pain, nausea, and vomiting. Perforations can be caused by the lymphoma itself or by its treatments.
If your doctor suspects that you have intestinal problems, they will recommend several tests that can determine whether there is an underlying disease. Those may include:
Intestinal lymphomas are also diagnosed using endoscopy. During an endoscopic procedure, a doctor passes a thin tube containing a camera through your mouth or your rectum. Another approach is to swallow a small pill-sized capsule that contains a camera. The camera collects images that can offer a closer look at intestinal tissue. Doctors may take a biopsy (remove a small piece of tissue) during the endoscopy. Then they carefully study the sample under a microscope and look for lymphoma cells.
An important part of diagnosis is determining the stage of a person’s disease. In lymphoma, staging helps doctors estimate someone’s outlook and know which treatments may be best, considering. Doctors may determine lymphoma stage using one of three staging systems.
Those stage classification systems include:
Each of these systems use slightly different methods to describe what areas of the body contain lymphoma cells. To stage GI lymphoma, doctors use imaging tests like endoscopy, ultrasounds, computed tomography (CT) scans, positron emission tomography (PET) scans, and magnetic resonance imaging (MRI).
Different subtypes of intestinal lymphoma are treated with different methods. People without any symptoms or with indolent (slow-growing) lymphoma may not need treatment right away. They may be able to wait until the disease gets worse and symptoms appear.
Two main kinds of doctors oversee GI lymphoma treatments. They are hematologist-oncologists (combination blood disorder and cancer specialists) and gastroenterologists (digestive system disorder specialists).
Surgery can help doctors both diagnose and treat intestinal lymphoma. Surgery used to be a more common treatment for intestinal lymphoma. However, as lymphoma medications have improved, doctors use surgery less frequently than they once did. Now surgery is often only used to treat problems such as perforation. The name of the procedure someone with intestinal lymphoma gets in order to remove part of their intestine is called a resection.
Doctors often use chemotherapy regimens (combinations of different drugs) to treat intestinal lymphoma. Possible regimens include:
In some cases, lymphoma may be refractory (it doesn’t go away after being treated). In other cases, the cancer may relapse (disappear and then return). Doctors often treat refractory or relapsed intestinal lymphoma with different chemotherapy regimens. One such regime possibility is called DHAP. It includes dexamethasone (sold as Decadron), high-dose cytarabine (also called ara-C, both sold as Cytosar-U), and cisplatin (formerly sold as Platinol).
People with intestinal T-cell lymphoma often live longer and have more time before a relapse when they use a treatment plan called the Newcastle regimen or IVE/MTX-ASCT. This treatment combines a stem cell transplant with the chemotherapy drugs ifosfamide (sold as Ifex), etoposide (also called VP-16, both sold as Etopophos), epirubicin (sold as Ellence), and methotrexate (sold as Rheumatrex and Trexall).
Stem cell transplantation is a procedure in which a person’s unhealthy stem cells (the cells that make new blood cells) are replaced with healthy ones. Intestinal lymphoma is generally treated with an autologous (self) stem cell transplant. During this procedure, doctors remove a person’s stem cells before giving them high doses of chemotherapy or radiation therapy. The aggressive treatment kills many of their lymphoma cells — and their healthy blood cells, too. Once the treatment is done, doctors place the “rescued” autologous stem cells back into the person’s body. Those healthy stem cells then create new blood cells.
In some cases, intestinal lymphoma may be treated with an allogeneic (donor) stem cell transplant. This procedure is largely the same as an autologous transplant, but the new stem cells come from a donor.
Targeted therapies are drugs that recognize and block specific molecules found on cancer cells. Rituxan (rituximab) is one targeted therapy sometimes given along with chemotherapy to treat intestinal lymphoma.
Researchers are studying other targeted therapy drugs for intestinal lymphoma in clinical trials. Ask your health care team if participating in one of these clinical trials is a good option for you.
Palliative treatments help ease the signs and symptoms of a chronic disease. The intestines are responsible for absorbing nutrients from food, so people with intestinal lymphoma may have problems getting proper nutrition. Getting food through a feeding tube or through an IV can help people with intestinal lymphoma get more nutrients.
A person’s prognosis (outlook) depends on how early their lymphoma was diagnosed. People with early-stage lymphoma generally have better outcomes than do people who are diagnosed with late-stage disease. Also, different intestinal lymphoma subtypes have different survival rates and relapse rates.
One Chinese study looked at 81 patients with intestinal lymphoma — most of whom had the B cell lymphoma subtype. Sixty-six of the total group were in stages 1 or 2. Based on the entire pool of 81, researchers determined more than 7 out of 10 of them lived for 5 years or more after being diagnosed and treated. And, the study also found, more than 6 out of 10 of those survivors lived without relapses.
Just as with other types of NHL, doctors determine a person’s intestinal lymphoma prognosis using an International Prognostic Index (IPI). Doctors use IPIs to come up with a score that describes a person’s risk level. People with a high-risk score are more likely to have worse outcomes than are people with a low-risk score. An IPI score is based on a person’s:
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Mark Levin, M.D.
is a hematology and oncology specialist with over 37 years of experience in internal medicine. Review provided by VeriMed Healthcare Network.
Learn more about him here.
Maureen McNulty
studied molecular genetics and English at Ohio State University.
Learn more about her here.
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So glad to have come across this article as there seems to be so little written about the condition.
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